Klippel-Trenaunay syndrome (KTW) is a rare, sporadic syndrome
characterized by a triad of port-wine stain, varicose
veins with or without venous malformations, and bony and soft tissue
hypertrophy. It usually affects only one extremity, organ involvement is uncommon.A case of 17-year-old boy with this syndrome having solid viscous organ involvement was observed at
surgery Outdoor. This case is rare so it was reported. Splenic
lymphangiomatosis is also observed in this KTW syndrome case as association of
AVM and lymphangioma together in spleen is rarest which was observed in this
case. So it is a very rare case.